Presentation
It averages a 90% mortality rate within 18 months of presentation. Brainstem Gliomas (BSGs) affect 200-300 children in the United States, mostly between the ages of 6 to 9 years, and are the leading cause of brain tumor death in children. [curesearch.org]
Median age at diagnosis is in the mid-30s, though brainstem gliomas can present in any decade of life. [ncbi.nlm.nih.gov]
Entire Body System
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Pain
Nettie and Brad were determined to raise awareness and badly needed funds for research so that no other family would have to endure the pain that they had just faced. [curesearch.org]
Chemotherapy in Treating Children With Progressive Brain Tumors Completed NCT00002944 Phase 3 carboplatin;lomustine;procarbazine hydrochloride;thioguanine;vincristine sulfate 6 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain [malacards.org]
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Weight Loss
Radiation Only in Diffuse, Intrinsic, Brainstem Glioma Not yet recruiting NCT02887040 Phase 3 Atengenal;Astugenal 11 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride [malacards.org]
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Hodgkin Lymphoma
Lymphoma Completed NCT00006102 Phase 2 becatecarin 30 Chemotherapy and Radiation Therapy After Surgery in Treating Children With Newly Diagnosed Astrocytoma, Glioblastoma Multiforme, Gliosarcoma, or Diffuse Intrinsic Pontine Glioma Completed NCT00028795 [malacards.org]
Respiratoric
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Pneumonia
Abstract A small fibrillary astrocytoma of the brain stem led to bulbar palsy, persistent regurgitation, and death from aspiration pneumonia in a 4-month-old boy. [pediatrics.aappublications.org]
Gastrointestinal
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Nausea
Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo 7 Donepezil in Treating Patients Who Have Undergone Radiation Therapy for Brain Tumors Completed NCT00369785 Phase 3 donepezil hydrochloride;Placebo 8 Acupressure in Controlling Nausea [malacards.org]
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Nausea
Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo 7 Donepezil in Treating Patients Who Have Undergone Radiation Therapy for Brain Tumors Completed NCT00369785 Phase 3 donepezil hydrochloride;Placebo 8 Acupressure in Controlling Nausea [malacards.org]
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Regurgitation
Abstract A small fibrillary astrocytoma of the brain stem led to bulbar palsy, persistent regurgitation, and death from aspiration pneumonia in a 4-month-old boy. [pediatrics.aappublications.org]
Psychiatrical
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Fear
“We were paralyzed with fear. I could hardly breathe,” recalls Nettie. “We could not believe this was happening. Julian had always been so healthy, so happy…so normal.” [curesearch.org]
Neurologic
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Headache
In 2010, Jeff Hurdle started experiencing headaches every few weeks. He’d never had frequent headaches before, so he ignored them. [mdanderson.org]
The most frequent presenting symptom for adult brainstem glioma is headache, which can be a manifestation of hydrocephalus. Cranial nerve deficits and long tract signs are also common. [ncbi.nlm.nih.gov]
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Babinski Sign
Clinical examination revealed multidirectional gaze-evoked nystagmus, bilateral Babinski sign, and gait and (left-predominant) limb ataxia. [jamanetwork.com]
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Nystagmus
Clinical examination revealed multidirectional gaze-evoked nystagmus, bilateral Babinski sign, and gait and (left-predominant) limb ataxia. [jamanetwork.com]
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Ataxia
Clinical examination revealed multidirectional gaze-evoked nystagmus, bilateral Babinski sign, and gait and (left-predominant) limb ataxia. [jamanetwork.com]
Treatment
Treatment Options For pediatric DIPG, standard of care treatment consists of involved field radiation therapy, typically to a dose of approximately 54–60 Gy. [ncbi.nlm.nih.gov]
The authors believe that this hypothesis, in conjunction with clinical data, may help establish selection criteria for the surgical treatment of specific brain-stem lesions. [thejns.org]
Completed NCT00091182 Phase 2 oxaliplatin 45 Cyproheptadine and Megestrol in Preventing Weight Loss in Children With Cachexia Caused By Cancer or Cancer Treatment Completed NCT00066248 Phase 2 cyproheptadine hydrochloride;megestrol acetate 46 Temozolomide [malacards.org]
Jeff didn’t want to travel for brain stem tumor treatment and leave his wife and two small children -- Kate, who was 3 years old at the time, and Logan, then just 4 months old. [mdanderson.org]
Prognosis
The authors propose an anatomical hypothesis that helps identify glioma growth patterns in general and that clarifies why cervicomedullary, dorsally exophytic, and focal tumors have a more favorable prognosis. [thejns.org]
His parents rushed him to the emergency room where a CT scan revealed a mass inside of his brain which was later diagnosed as a brain stem glioma, a form of brain cancer with an extremely poor prognosis. [curesearch.org]
Location also factors into prognosis – the MD Anderson study demonstrated a median survival of 66.0 months for midbrain tumors, 25.3 months for pontine tumors, and 51.3 months for medullary tumors ( 5 ). [ncbi.nlm.nih.gov]
Progressive Gliomas Completed NCT00002749 Phase 2 carboplatin 37 Temozolomide in Treating Patients With Progressive Low-Grade Glioma Completed NCT00003466 Phase 2 temozolomide 38 Irinotecan in Combination With Cisplatin in Pediatric Patients With Unfavorable Prognosis [malacards.org]
Etiology
Within the umbrella of adult brainstem glioma, there is a subset of tumors that is similar in appearance and potentially similar in etiology to childhood DIPG, but with slightly older onset, generally affecting young adults. [ncbi.nlm.nih.gov]
Pathophysiology
As befits a diagnosis that encompasses an array of pathophysiologic alterations, the radiographic appearance of adult brainstem glioma varies widely, with approximately 40% demonstrating enhancement ( 3, 5 ). [ncbi.nlm.nih.gov]
Prevention
Radiation Only in Diffuse, Intrinsic, Brainstem Glioma Not yet recruiting NCT02887040 Phase 3 Atengenal;Astugenal 11 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride [malacards.org]